Temporary response of localized intracranial mast cell sarcoma to combination chemotherapy

Cerebral involvement of systemic mastocytosis and intracranial sarcoma of myelogenic origin are well known entities. An 8-year-old girl with an isolated cerebral mast cell tumor is presented. Specific histopathologic stains were used to confirm the diagnosis detecting immunophenotype and proliferative activity. Treatment with irradiation, intrathecal cytarabine, and interferon-alpha2b did not induce regression whereas polychemotherapy did. Systemic combination chemotherapy led to marked transient tumor regression in this proliferating mast cell sarcoma in an unusual intracranial location.     

Acquired neuropathologic lesions associated with the hypoplastic left heart syndrome

This study details the type, frequency, clinical presentation, and etiologic associations of acquired brain lesions in 40 infants with the hypoplastic left heart syndrome encountered during a 52-month interval. Detailed postmortem neuropathologic examinations showed that 55% of the infants were free of acquired brain lesions. However, the other 45% had combinations of hypoxic-ischemic lesions and intracranial hemorrhage. Central nervous system perfusion and glucose-oxygen delivery appeared to be important factors in the occurrence of hypoxic-ischemic lesions or intracranial hemorrhage, whereas acidosis and hypercarbia were not. Cerebral necrosis may be a predisposing factor for a major intracranial hemorrhage. A duration of cardiopulmonary bypass with hypothermic total circulatory arrest longer than 40 minutes was associated with a higher incidence of acquired neuropathology. These results indicate that the majority of infants with hypoplastic left heart syndrome are free of acquired neuropathology and suggest practical ways to reduce the risks in the others.     

Ruptured middle cerebral artery aneurysm with intramural myxoid degeneration in a child

A 9-year-old girl presented with a subarachnoid hemorrhage. Cerebral angiography showed a saccular aneurysm arising from the horizontal portion of the right middle cerebral artery. The aneurysm was successfully clipped, and the dome of the aneurysm was excised for pathological study. Histological examination of the aneurysm wall showed that the entire thickness of the wall showed an increased myxoid degeneration. No dissection was present. Most intracranial aneurysms in childhood are believed to be of the saccular type similar to that in adults, but the pathogenesis of the aneurysm formation remains controversial. Myxoid degeneration may cause intracranial saccular aneurysm with eventual rupture, even in the absence of dissection. This is the first case reported of a ruptured saccular aneurysm caused by myxoid degeneration in a child. The possible pathophysiology is discussed.     

Tuberculous meningitis complicated with hydrocephalus and cerebral salt wasting syndrome in a three-year-old boy

Cerebral salt wasting syndrome (CSWS) is a syndrome of hyponatremia and natriuresis described in patients with intracranial diseases. We describe a 3-year-old boy with tuberculous meningitis complicated by hydrocephalus and CSWS and emphasize the different clinical presentation and management of patients with CSWS and the syndrome of inappropriate secretion of antidiuretic hormone.     

Colour Doppler imaging of intracranial vasculopathy in severe infantile sialidosis

Neonatal ascites is usually attributed to prenatal infections, lysosomal storage disease and anomalies of the genitourinary tract, gastrointestinal tract or cardiovascular system. We report one case of neonatal ascites associated with infantile sialidosis. Cerebral sonography showed stripe-like intracerebral echogenicities in the region of the basal ganglia. Colour Doppler imaging demonstrated blood flow within the echogenicities confirming the suspected diagnosis of intracranial vasculopathy.     

Calcified Cerebral Cryptococcal Granuloma

The authors report a 12-mo-old girl with calcified cerebral cryptococcal granuloma. She was admitted with a 6-mo history of seizures. Laboratory examinations showed no abnormal findings. Electroencephalography revealed bilateral slow wave activity, greater in the right occipital region. CT showed an irregular calcified focus with small surrounding low density in the right parieto-occipital region. MRI demonstrated mixed signals without edema and visible flow-voids. The clinical symptoms mimicked intracranial vascular malformations. The diagnosis of cerebral cryptococcal granuloma was made by histopathology. Partial resection of the lesion with post-operatively antifungal and anticonvulsant therapy offered the satisfactory result. Cerebral cryptococcal granuloma is extremely rare, especially in infants. Calcification is indeed unusual. Cerebral cryptococcal granuloma should be included in the differential diagnosis of intracranial mass with calcification in infants.     

Brain swelling in the asphyxiated term newborn: pathogenesis and outcome

The role of brain swelling following acute hypoxic-ischemic insult in the genesis of brain injury in the term newborn is controversial. Recent experimental animal studies suggest that it may result from prior irreversible cerebral necrosis and therefore represents a consequence as opposed to a cause of major brain injury. In this study, 32 asphyxiated term newborns were studied during the first week of life with serial intracranial pressure measurements. A total of 26 infants had CT scans during the first five days of life. Seven patients had two CT scans within this period. These investigations were correlated with outcome at 18 months of age. Seven infants had increased intracranial pressure (greater than 10 mm Hg) that reached a maximum between 36 and 72 hours of age. Cerebral perfusion pressures remained normal, which makes ongoing ischemic injury unlikely as a cause. The seven patients with increased intracranial pressure had decreased attenuation on CT that was generalized in six infants and patchy in one infant. Of the infants with increased intracranial pressure and severe CT abnormalities, three died and four had severe neurologic sequelae. In seven infants, a second CT scan at three to four days of life demonstrated progression of the decrease in tissue attenuation. Most of the infants with normal intracranial pressure (23/25) had no or had only minor neurologic abnormalities at follow-up. These data suggest that brain swelling is relatively uncommon in the asphyxiated term newborn.(ABSTRACT TRUNCATED AT 250 WORDS)     

Cerebral arteriovenous malformations in a probable familial form of Rendu-Osler disease]

Cerebral arteriovenous malformations with neonatal manifestations are infrequent and virtually always fatal. Heart failure with an intracranial bruit is the most common presentation. Exceptionally, the aneurysm is a manifestation of Rendu-Osler-Weber syndrome which is inherited on an autosomal dominant basis. Development of cerebral arteriovenous malformations occurs very early as demonstrated by the discovery of two aneurysms with major repercussions on the cerebral parenchyma in a female with severe prematurity. Pregnant women with suspected Rendu-Osler-Weber syndrome should undergo ultrasound studies targeted at identifying untreatable cerebral lesions antenatally.     

Resuscitation of severe meningitis in infants and children

Meningeal penetration of bacteria induces an inflammatory response which affects mainly the endothelium of cerebral vessels. This inflammatory reaction is directly responsible for thrombosis and indirectly creates cerebral oedema and reduced cerebral blood flow. Cerebral ischemia is the result of all these phenomenons. Nowadays, control of cerebral oedema is the main goal of intensive care treatment of meningitis that needs invasive monitoring of intracranial pressure. We may hope that in a next future new therapeutic agents will be able to control inflammatory reaction and vascular process.     

Newborn infants with a birth weight of 1000 g and less. A regional study of 124 cases]

A retrospective and regional survey was performed in 1986 and 1987 in the neonatal centers of western France (9 districts of Pays de Loire and Bretagne with 156,134 births). The epidemiology of 124 neonates with a birth weight of 1,000 g or less was analysed and the follow-up was studied at the age of two. The incidence was 0.8 per thousand births. The mortality was 44.3%. There were no differences between surviving and dead infants concerning obstetrical circumstances, presentation and mode of delivery, multiple or single pregnancies and birth place. Seventy per cent were incubated in the delivery room. Forty-five per cent had respiratory distress syndrome (RDS): 60% of them died (vs 30% without RDS; P less than 0.001) 41% had ultrasound intracranial anomalies (vs 8.5% without RDS; P less than 0.003). There was significantly less death when the duration of rupture of membranes was greater than 12 hours (28% vs 51.7% for a duration less than 12 hours; P less than 0.03). Cerebral palsy was present in 19.3% of the survivors followed until the age of 2 years. The disabled infants differed significantly from normal infants in term of RDS and ultrasound intracranial anomalies. From this study, the incidence of infants with very low birth weight appears low in western France, possibly related to the fact that infants died before birth or before being sent to neonate units; survival and sequelae rate is comparable with other studies.     

颅内出血早产儿生后四天内脑血流变化及围生因素分析

目的　探讨颅内出血早产儿生后 4d内脑血流动力学的变化及颅内出血与围生因素的关系。方法　应用经颅多谱勒超声 (TCD)检测 2 9例颅内出血早产儿脑血流速度、阻力指数的变化及脑血流速度与平均动脉血压的关系 ,分析临床围生相关因素 ,并与同期无颅内出血的 2 8例早产儿进行比较。结果　颅内出血组早产儿于生后 2 4、4 8、72h脑血流速度增快 ,出生 2 4h平均脑血流速度与平均动脉血压相关 ,且与出生窒息、胎龄、出生体重、高PaCO2 有关。结论　颅内出血早产儿于生后 2 4h内存在脑血流自主调节功能破坏 ,生后 3d脑血流存在过度灌注 ;且出生窒息 ;胎龄 <32周、出生体重 <15 0 0 g及PaCO2 >6 6 7kPa为颅内出血的高危因素。     

Hemorrhagic stroke associated with pulmonary edema and catastrophic cardiac failure

Cerebral arteriovenous fistula (AVF) is a vascular malformation that is rare in the pediatric population. Older children with cerebral AVF tend to present with neurologic problems related to intracranial venous hypertension or intracranial hemorrhage. Cardiac and pulmonary complications following acute neurologic injury such as subarachnoid hemorrhage are common in adults, but are rarely reported in children. However, complications have been reported in cases of enterovirus 71 rhombencephalitis in infants and children and can cause high morbidity and mortality. Here, we report a 14-year-old boy who presented with cardiac failure associated with pulmonary edema following cerebral hemorrhagic stroke due to AVF. After aggressive investigation and management, we intervened before significant hypoxia and hypotension developed, potentially reducing the risk of long-term adverse neurologic consequences in this patient.     

Cranial MR venography findings of severe hypernatremic dehydration in association with cerebral venous thrombosis in the neonatal period

Severe neonatal hypernatremia is an important electrolyte disorder that has serious effects on the central nervous system, including brain edema, intracranial hemorrhage, hemorrhagic infarct, and thrombosis. Cerebral venous thrombosis is relatively rare in severe neonatal hypernatremic dehydration. The English literature contains only a few reports of the cranial radiological findings in severe neonatal hypernatremia. The authors report cranial MR venography findings of a newborn infant with severe hypernatremic dehydration. To the best of their knowledge, this is the first such report in the English literature.     

Syndrome d'Angelman et anévrisme intracrânien : association fortuite ou prédisposition génétique commune ?

Although the pathogenesis of cerebral aneurysms has been studied intensively, it is yet poorly understood. However, a genetic predisposition to this pathology has been often suspected. We describe a patient with both intracranial aneurysm and Angelman syndrome. Angelman syndrome is characterized by severe mental retardation, inappropriate laughter, absent speech, dysmorphic facial features and seizures. It is due to genetic abnormalities of chromosome 15. Cerebral aneurysms are sometimes associated with inherited diseases like autosomal dominant polycystic kidney disease. Moreover several candidate genes have been analysed, to search for genetic variants which might be associated with the occurence of intracranial aneurysms. Our question is: is the association described in our observation fortuitous or do these diseases share a same genetic predisposition? Our observation also supports the hypothesis of a genetic participation in the genesis of cerebral aneurysms.     

CRANIAL MR VENOGRAPHY FINDINGS OF SEVERE HYPERNATREMIC DEHYDRATION IN ASSOCIATION WITH CEREBRAL VENOUS THROMBOSIS IN THE NEONATAL PERIOD

Severe neonatal hypernatremia is an important electrolyte disorder that has serious effects on the central nervous system, including brain edema, intracranial hemorrhage, hemorrhagic infarct, and thrombosis. Cerebral venous thrombosis is relatively rare in severe neonatal hypernatremic dehydration. The English literature contains only a few reports of the cranial radiological findings in severe neonatal hypernatremia. The authors report cranial MR venography findings of a newborn infant with severe hypernatremic dehydration. To the best of their knowledge, this is the first such report in the English literature.     

Reye's syndrome. A current entity which remains enigmatic

Reye's syndrome (RS) has been defined by the CDC as an acute non-inflammatory encephalopathy and hepatopathy, with no reasonable explanation for the cerebral and hepatic abnormalities. The diagnosis of RS is made by a constellation of clinical and laboratory features, none of which are pathognomonic. The demonstration of a fatty infiltration on liver biopsy is not really specific. On the other hand, the mitochondrial ultrastructural abnormalities appear to be characteristic of RS. Cerebral edema with intracranial hypertension represents the most immediate threat to life, the cause of the encephalopathy being unknown. The treatment of RS consists in perfusion of hypertonic glucose solution and management of intracranial hypertension. Grade I RS should be recognized early and treated with intravenous glucose. A number of metabolic disorders may yield a clinical picture resembling RS. These include disturbances of organic acid metabolism, urea cycle defects, and disorders of carbohydrate metabolism. The pathogenesis of RS is a mitochondrial insult induced by different viruses, drugs, exogenous toxins, and genetic factors. In the USA, pilot studies showed that a majority of RS cases may be attributable to salicylate use. In France, RS remains unfrequent although no precise epidemiological data are available. An epidemiological study appears therefore necessary for a better understanding of this mysterious syndrome. There is a need for a biological marker of the mitochondrial injury.     

The effects of intermittent positive pressure ventilation on cerebral arterial and venous blood velocities in the newborn infant

Cerebral arterial and venous blood velocities were measured using pulsed Doppler ultrasound in 25 newborn infants requiring intermittent positive pressure ventilation (IPPV). The aim was to investigate whether these velocities altered in relation to the peak inflation pressure (PIP) used. Continuous recordings were made on the superior sagittal sinus and an intracranial artery both at the clinically prescribed PIP, and while this was altered in steps. A majority of the infants had, at some stage of their illness, variations in their venous and arterial velocities in phase with IPPV and related to the PIP used. These variations could be reduced by lowering the PIP. This was done in 5 infants whilst we were still able to maintain adequate ventilation.     

The Effects of Intermittent Positive Pressure Ventilation on Cerebral Arterial and Venous Blood Velocities in the Newborn Infant

ABSTRACT. Cerebral arterial and venous blood velocities were measured using pulsed Doppler ultrasound in 25 newborn infants requiring intermittant positive pressure ventilation (IPPV). The aim was to investigate whether these velocities altered in relation to the peak inflation pressure (PIP) used. Continous recordings were made on the superior sagittal sinus and an intracranial artery both at the clinically prescribed PIP, and while this was altered in steps. A majority of the infants had, at some stage of their illness, variations in their venous and arterial velocities in phase with IPPV and related to the PIP used. These variations could be reduced by lowering the PIP. This was done in 5 infants whilst we were still able to maintain adequate ventilation.     

Cerebral sinus venous thrombosis in children

OBJECTIVE: Cerebral sinus venous thrombosis (cerebral SVT) is rare in children. Information on clinical characteristics, radiological findings and outcome is emerging. METHODS: Cases of cerebral SVT diagnosed between 1995 and 2001 were identified by a computer-assisted search using International Classification of Disease codes. Medical records were reviewed to collect information on clinical presentation, investigations, treatment and outcome. RESULTS: Sixteen cases of cerebral SVT were identified. All cases presented in association with head and neck pathology. The majority of cases presented with symptoms of raised intracranial pressure and focal neurological signs. Magnetic resonance imaging identified all cases of cerebral SVT whilst CT scanning failed to demonstrate the diagnosis in two cases. Management with anticoagulation was associated with radiological resolution of the thrombosis and normal neurological outcome. Long-term follow up demonstrated neurological deficits in greater than 40% of patients. CONCLUSION: Cerebral SVT in children is associated with significant residual neurological morbidity. Prospective studies to identify predictors of outcome and effective management interventions are required.     

Cerebral metastasis and other central nervous system complications of pleuropulmonary blastoma

BACKGROUND: Pleuropulmonary blastoma (PPB) is a rare tumor of pleura and lung in young children. Central nervous system (CNS) complications, particularly cerebral parenchymal metastases, occur in aggressive forms of PPB: Types II and III PPB. This article evaluates cerebral and meningeal metastases, cerebrovascular events (CVA) caused by tumor emboli, spinal cord complications, and intracranial second malignancies in PPB. PROCEDURE: International PPB Registry and literature cases were evaluated for CNS events. Cerebral metastasis patients were evaluated for gender, side of origin of PPB, PPB Type, interval from diagnosis to metastasis, status of chest disease, treatment, and outcome. Standard statistical methods were used to calculate the cumulative probability of cerebral metastasis and survival following metastasis. RESULTS: Thirty-nine cases of cerebral metastasis were identified in 5/53 Registry Type II cases, 15/44 Registry Type III cases, and 19/143 literature Type II/III cases. Metastases occurred 1-60, median 11.5 months after diagnosis. Chest disease was controlled in 50% of children at time of metastasis. The cumulative probability of cerebral metastasis by 5 years from diagnosis was 11% for Type II patients (95%CI (confidence interval): 2-20%) and 54% for Type III patients (95%CI: 31-76%). Seven children survive cerebral metastasis. Other CNS complications were post-operative CVA (five cases), spinal cord invasion or compression (six), leptomeningeal disease (three), and second intracranial malignancies (two). CONCLUSIONS: Cerebral metastasis is more frequent in PPB than in other childhood sarcomas. Clinicians should screen for this complication. Diverse other CNS complications are less common and require careful diagnosis.